Infliximab treatment for severe granulomatous disease in common variable immunodeficiency: a case report and review of the literature
Infliximab treatment for severe granulomatous disease in common variable immunodeficiency: a case report and review of the literature
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DOI：
10.1016/s1081-1206(10)61228-8
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发表时间：
2005-09-01
影响因子：
5.9
通讯作者：
White, AJ
中科院分区：
文献类型：
--
作者：
Thatayatikom, A;Thatayatikom, S;White, AJ
背景：类似结节病的肉芽肿性疾病是一种与常见变异型免疫缺陷（CVID）相关的疾病。其治疗仍然存在问题，需要新的治疗选择。目的：报告使用英夫利西单抗（一种嵌合抗肿瘤坏死因子单克隆抗体）治疗肉芽肿性CVID患者的疗效，并回顾肉芽肿性CVID患者治疗的文献。一名患有CVID的22岁白色男性发生急性多器官衰竭，肺和肝活检标本上有肉芽肿性炎症。他最初接受抗生素、静脉注射免疫球蛋白和皮质类固醇治疗5周，无改善。高剂量英夫利西单抗每周输注6周，然后每月输注9个月。对英夫利西单抗的反应是由临床检查，影像学研究和组织学研究的变化确定的。结果：患者的病情显着改善后，1dose的英夫利西单抗输注，减少肝脾肿大，辅助支持的要求，和肺浸润。在3周内成功停止通气支持。皮质类固醇剂量逐渐减少，没有疾病复发。治疗9个月后，随访影像学研究显示肺浸润消退，无肝脾肿大，无门脉高压，经皮肝活检显示无肉芽肿;然后，停用英夫利西单抗。患者仍然免费的肉芽肿性疾病后18个月的follow-up.Conclusions：据我们所知，这是严重的内脏肉芽肿性CVID成功治疗英夫利西单抗的第一份报告。英夫利西单抗可能是治疗CVID肉芽肿的有效方法。需要进一步研究英夫利西单抗和其他肿瘤坏死因子α拮抗剂治疗肉芽肿性CVID。
Background: Granulomatous disease resembling sarcoidosis is a well-described condition associated with common variable immunodeficiency (CVID). Its treatment remains problematic, and new therapeutic options are needed.Objectives: To report the efficacy of treatment with infliximab, a chimeric anti-tumor necrosis factor a monoclonal antibody, in a patient with granulomatous CVID and to review the literature on the treatment of patients with granulomatous CVID.Methods: A 22-year-old white man with CVID developed acute multiorgan failure, with granulomatous inflammation on lung and liver biopsy specimens. He was initially treated with antibiotics, intravenous immunoglobulin, and corticosteroids for 5 weeks without improvement. High-dose infliximab was then infused weekly for 6 weeks and then monthly for 9 months. The response to infliximab was determined by changes on clinical examination, imaging studies, and histologic studies.Results: The patient's condition dramatically improved after 1dose of infliximab infusion, with decreasing hepatosplenomegaly, ventilatory support requirements, and pulmonary infiltrates. Ventilatory support was successfully discontinued within 3 weeks. The corticosteroid dose was tapered without reactivation of the disease. After 9 months of therapy, follow-up imaging studies showed resolution of pulmonary infiltrates, no hepatosplenomegaly, and no portal hypertension, and a percutaneous liver biopsy revealed no granulomas; then, infliximab use was discontinued. The patient remains free of granulomatous disease after 18 months of follow-up.Conclusions: To our knowledge, this is the first report of severe visceral granulomatous CVID successfully treated with infliximab. Infliximab may be an effective therapy for granulomas in CVID. Further studies of infliximab and other tumor necrosis factor a antagonist therapies in granulomatous CVID are warranted.